Comprehensive mastocytosis data analysis from a single center.

Mastocytosis is a very rare disorder and is divided into three prognostically distinct variants by World Health Organization: Cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma or localized mast cell (MC) tumors. The wide range of complaints may cause patients to consult various clinics, with resulting mis- or underdiagnosis. Therefore, cooperation between different subspecialties is of paramount importance. In this article, we have compiled 104 adult mastocytosis cases diagnosed and followed in our Hematology and other clinics. 86 (82.7%) of 104 patients had systemic mastocytosis. Osteoporosis, disease-related complications, and secondary malignancies are important topics in this group. We know that indolent form has great survival. But smoldering or aggressive mastocytosis has a poor prognosis. CM and indolent SM have a significantly better prognosis compared to aggressive SM (p < 0.001). We found that the presence of more than 25% of mast cells in the bone marrow, the presence of concomitant marrow dysplasia, and the presence of disease-related complications affect survival (p < 0.001). In addition to the WHO classification, the IPSM scoring system is indicative of the prognosis in this rare disease.

Biosimilar Rituximab (Redditux) Added to CHOP Chemotherapy for De Novo Diffuse Large B-Cell Lymphoma Patients: Real-Life Single-Center Experience

Objective: Redditux® (RED), as a biosimilar rituximab, was approved in Turkey for all indications of the original Mabthera® (MAB) in March 2018. The aim of our study was to evaluate the efficacy and safety of RED in de novo diffuse large B-cell lymphoma. Materials and Methods: Fifty-one patients received RED combined with the CHOP regimen. The median follow-up was 31 months. The historical control group included 219 patients treated with the MAB-CHOP regimen and the median follow-up time was 38 months. We compared the response rates and survival outcomes of these RED-CHOP and MAB-CHOP cohorts. Results: In the RED cohort, the overall response rate (ORR) at the end of the treatment protocol was 86%, with 37 (72.5%) cases of complete response (CR) and 7 (13.5%) cases of partial response (PR). In the historical MAB cohort, the ORR was 84%, with CR and PR rates of 82% and 2%, respectively. The 24-month progression-free survival (PFS) rates were 73.76% (95% confidence interval [CI]: 0.59-0.84) and 85.2% (95% CI: 0.79-0.90) for the RED and MAB cohorts, respectively (p=0.0106). The 24-month overall survival rates were 78.4% (95% CI: 0.64-0.87) and 81.4% (95% CI: 0.75-0.86) for the RED and MAB cohorts, respectively (p=0.7461). For patients with high revised International Prognostic Index scores, 24-month PFS was 45.5% (95% CI: 0.17-0.71) and 63% (95% CI: 0.37-0.80) for the RED and MAB cohorts, respectively (p=0.0711). In the RED cohort, central nervous system (CNS) relapse was significantly increased compared to the MAB cohort (10% vs. 1.83%, p=0.004). Among the RED cohort, bone involvement at the time of diagnosis was a risk factor for CNS relapse (p=0.028). Thirteen patients died in follow-up. There were no serious adverse events causing the cessation of the drugs. Conclusion: RED has an ORR similar to that of MAB. However, PFS rates were worse in the RED cohort. Additionally, CNS relapse ratio was a major concern for our RED cohort. Large prospective controlled studies and real-life data with longer follow-up are needed to document the non-inferiority of RED compared to MAB.

[A Case of Chronic Q Fever Endocarditis Mimicking Lymphoproliferative Disorders]. / Lenfoproliferatif Hastaliklarla Karisan Bir Kronik Q Atesi Endokarditi Olgusu.

Q fever is a zoonosis caused by Coxiella burnetii. In this report, a case of chronic Q fever endocarditis with pancytopenia and hypergammaglobulinemia mimicking a lymphoproliferative disease was presented. A 39-years-old male living in Çatalca and whose family is engaged in animal husbandry admitted with the complaints of weakness and fatigue. The patient had aortic valve replacement 29 years ago and had aortic valve re-replacement, and ascending aorta grafting because of endocarditis three years ago. It was revealed that the second operation of the patient was due to possible infective endocarditis, but no definitive agent could be identified. He was evaluated for massive hepatosplenomegaly, pancytopenia, hypergammaglobulinemia, presence of M-spike and elevated ß-2 microglobulin levels and was referred to our hematology clinic with a preliminary diagnosis of lymphoproliferative disease. Lymphoplasmacytic lymphoma was excluded with the result of bone marrow biopsy and he was referred to our clinic for the investigation of possible infectious etiologies. We detected hepatosplenomegaly and finger clubbing. His blood analyses were normal except for the following: leukocyte count 3800/µl, platelet count 148000/µl, gamma globulin 5.9 gr/dl, rheumatoid factor (RF) and antinuclear antibody (ANA) positivity. Chronic Q fever endocarditis was suspected and C.burnetii Phase I IgG test was found positive in 1/132071 titers. Although transesophageal echocardiography showed no lesion of endocarditis, positron emission tomography/computed tomography revealed increased fluorodeoxyglucose uptake around the prosthetic heart valve and graft. The patient was diagnosed as having Q fever endocarditis and graft infection. He refused hospitalization and was started on hydroxychloroquine and doxycycline treatment. The patient stopped taking these antibiotics by himself seven days after the diagnosis. He was admitted with a headache to another hospital and operated for an intracranial hemorrhage and died shortly after. Apart from unfamiliarity, wide range of clinical presentations of disease could also lead to delayed diagnosis. Among patients with chronic Q fever, continuous bacteremia and antigenic stimulus causes inflammatory syndrome with hepatosplenomegaly, hypergammaglobulinemia and, presence of autoantibodies which leads to misdiagnoses of rheumatologic, autoimmune or hematologic diseases Chronic Q fever should be investigated in patients with known valvulopathy and chronic hepatomegaly or splenomegaly, pancytopenia, hypergammaglobulinemia, and unexplained autoantibody positivity.

COVID-19 infection in patients with acute leukemia; Istanbul experience.

Coronavirus disease 2019 (COVID-19) has led to a global pandemic that has also challenged the management of various other life-threatening conditions, such as malignant disorders. In this study, we present the clinical features and treatment outcomes of twenty-seven COVID-19 positive patients with leukemia across seven different centers in Istanbul. From March 1st to December 31st 2020, 116 patients were diagnosed with acute leukemia. Thirty-two cases with acute lymphocytic leukemia (ALL), 82 cases with acute myeloid leukemia (AML), and 2 cases with mixed phenotype acute leukemia (MPAL) were identified. Of the 27 patients with the COVID-19 infection, seven patients had ALL, 19 patients had AML and one patient had MPAL. The mortality rate was 37% among the patients with AML, whereas there were no deaths in the ALL group. The mortality rate of AML patients with the COVID-19 infection was higher compared to cases without the infection (P<0.05). We could not detect any significant difference in the ALL cohort. This study, which includes one of the largest acute leukemia series in literature proved that acute myeloid leukemia patients with the COVID-19 infection have worse outcomes than patients without the infection. The high mortality among patients with acute leukemias hospitalized with COVID-19 highlight the need for aggressive infection prevention, increased surveillance and protective isolation and even modification of the therapy, in case of minimal residual disease (MRD) negativity.

Possible relationship between the resistin gene C-420G polymorphism and colorectal cancer in a Turkish population.

BACKGROUND/AIMS: In recent years, with improvements in genotyping, a possible relationship between obesity-related gene polymorphisms and colorectal cancer (CRC) has been studied. The promoter region C-420G of the resistin gene is believed to have an important role in the development of malignancy. We prospectively evaluated the possible effect of the resistin C-420G polymorphism on the risk and prognosis of CRC. MATERIALS AND METHODS: One hundred twenty-three patients with CRC and 79 healthy individuals were included in the study. Blood samples were genotyped, and the relationship between the resistin C-420G polymorphism and demographic characteristics and tumor features was evaluated. RESULTS: No statistically significant difference was found in genotype distribution between the patient and control groups and among patients in the means of gender, biochemical findings, and tumor characteristics (p>0.05). CONCLUSION: The relationship between the C-420G polymorphism and various diseases has been evaluated in many studies to date. With the increased importance of obesity in etiopathogenesis, studies have focused on CRC. According to our results, the GG genotype may be associated with a decreased CRC risk. Our study is important because to our knowledge, it is the first one to be conducted in a Turkish population to date, but we believe that more patients and controls are needed to obtain statistically significant results.

Can Positron Emission Tomography and Computed Tomography Be a Substitute for Bone Marrow Biopsy in Detection of Bone Marrow Involvement in Patients with Hodgkin's or Non-Hodgkin's Lymphoma?

INTRODUCTION: Positron emission tomography and computed tomography (PET/CT) has become an important part of staging and treatment evaluation algorithms of lymphoma. We aimed to compare the results of PET/CT with bone marrow biopsy (BMB) with respect to bone marrow involvement (BMI) in patients with Hodgkin's lymphoma (HL) and aggressive non-Hodgkin's lymphoma (aNHL). METHODS: The medical files of a total of 297 patients diagnosed with HL or aNHL and followed at the hematology clinics of 3 major hospitals in Istanbul between 2008 and 2012 were screened retrospectively and 161 patients with classical HL and aNHL were included in the study. The patients were referred for PET/CT and BMB at the initial staging. BMB was performed as the reference standard for the evaluation of BMI. RESULTS: There were 61 (38%) HL and 100 (62%) aNHL patients. Concordant results were revealed between PET/CT and BMB in 126 patients (78%) (52 HL, 74 aNHL), 20 with positive PET/CT and BMB results and 106 with negative PET/CT and BMB results. There were discordant results in 35 patients (9 HL, 26 aNHL), 16 of them with positive BMB and negative PET/ CT results and 19 of them with negative BMB and positive PET/CT results. DISCUSSION AND CONCLUSION: We observed that PET/CT is effective to detect BMI, despite it alone not being sufficient to evaluate BMI in HL and aNHL. Bone marrow trephine biopsy and PET/CT should be considered as mutually complementary methods for detection of BMI in patients with lymphoma. In suspected focal involvement, combining biopsy and PET/CT might improve staging results.

A rare entity in multiple myeloma: six nerve paralysis.

Ophthalmic involvement appears rarely in multiple myeloma (MM). Ophthalmic findings are mostly noted as complications caused by disease or treatment. MM-associated with 6th nerve paralysis is a rare entity. Bortezomib, one of the novel agents used to treat MM, has neurotoxic effect and may cause permanent nerve damage. Herein, we report a 50-year-old male patient with MM who developed the 6th nerve paralysis while receiving bortezomib and discuss its relevant causes.